Sickle Cell Disease and Bartonella Spp. Infection

Paulo Eduardo Neves Ferreira Velho1, Marna Elise Ericson2, David Mair3 and Kalpna Gupta4

1Division of Dermatology, Department of Medicine, University of Campinas / UNICAMP, Brazil and Research Associate of the Department of Dermatology, University of Minnesota, USA
2Department of Dermatology / Center for Drug Design, University of Minnesota, USA
3American Red-Cross-North Central Region, USA
4Division of Hematology, Oncology and Transplantation, Department of Medicine, University of Minnesota, USA

Correspondence to: Paulo Eduardo Neves Ferreira Velho; M.D., Ph.D. Assistant Professor of the Division of Dermatology, Department of Medicine, University of Campinas / UNICAMP, Brazil and Research Associate of the Department of Dermatology, University of Minnesota, USA. 420 Delaware St. S.E., MMC98, Minneapolis, MN, USA 55455. Tel: 612-625-6707, Fax: 612-624-6678. E-mail:;

Published: June 30, 2012
Received: May 16, 2012
Accepted: June 6, 2012
Medit J Hemat Infect Dis 2012, 4(1): e2012046, DOI 10.4084/MJHID.2012.046
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This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited

To the Editor,
We read with great interest the recent article The role of infection in the pathogenesis of vaso-occlusive crisis in patients with sickle cell diseases, written by Dr. Ahmed SG. He pointed that these patients have impaired immunity and are thus predispose to infections which can precipitate the painful crisis.[1] SCD is the most common hereditary hematologic disorder in the world and remains a significant global health problem with high relevance to low- and middle-income countries.[2] The vast majority of SCD patients live in underdeveloped nations with high prevalence and transmission rates of infections.[1] In equatorial Africa 10-40% of native populations have sickle (S) gene.[2] In Brazil some afro-descendent groups have a prevalence of up to 10% of S gene and the disease is a relevant public health problem.[2,3] SCD patients have infections which are often asymptomatic.[1] Gram-negative infection are frequent in pneumococcal vaccineted SCD patients that had been splenectomized and functional asplenia is as frequent as 90% by 6 years of age.[4]
These patients frequently need blood transfusion and transmission of pathogens via transfusion in SCD patient infection has been documented.[5] Amongst the potential gram-negative infections Bartonella spp. are emergent bacteria with worldwide distribution. An increasing number of Bartonella spp. are regarded as zoonotic pathogens, creating a public health concern for human and veterinary medicine. The extent of Bartonella spp. infection is underestimated.[6] Bartonella spp. bacteremia is potentially fatal, especially in immunodeficient patients. Immunocompetent individuals are also at risk for chronic infection by this intra-erythrocyte and intra-endothelial agent though the infection can be asymptomatic.[7] A broad spectrum of clinical manifestations have been related to Bartonella spp. infection, many of which were considered idiopathic prior to the diagnosis of chronic Bartonella spp. infection. A recent study from the United States of America found that almost 50% of patients with non-specific symptoms (fatigue, sleeplessness, joint and muscle pain etc.) had positive Bartonella spp. serology and/or blood PCR positive to Bartonella spp. One in four patients had Bartonella spp. bacteremia.[8]
Previous studies show that blood donors can have asymptomatic bacteremia.[7] There are no gold-standard tests to confirm Bartonella spp. infection and false-negative results are frequent even with serology and multi-step molecular and microbiological techniques.[8] Thus diagnostic tests for Bartonella spp. remains challenging, warranting development of more sensitive and reproducible diagnostic methods.
It is likely that SCD patients could have a higher prevalence of Bartonella spp. infection rate as they present with inflammation, endothelial activation, asplenia, and the need for frequent blood transfusions; pathological features that can promote the invasion and progression of Bartonella spp..[1,4,6] Pain, fatigue and fever, characteristic features of Bartonella spp. infection are manifest in SCD. Therefore, coordinated international efforts should be initiated to evaluate the relevance of this infection in SCD and other chronic immunodeficient patients.


  1. Ahmed SG. The role of infection in the pathogenesis of vaso-occlusive crisis in patients with sickle cell disease. Mediterr J Hematol Infect Dis. 2011;3: e2011028.  PMid:21869914 PMCid:3152450  
  2. World Health Organization. Sickle Cell Anaemia. 59th World Health Assembly Report. A59/9, Geneva, 2006.
  3. Canšado RD, Jesus JA. Sickle cell disease in Brazil. Rev. bras. hematol. hemoter. 2007;29:203-206.  
  4. Driscoll MC. Sickle cell disease. Pediatrics in Review. 2007;28: 259-267. PMid:17601938  
  5. Slavov SN, Kashima S, Pinto AC, Covas DT. Human parvovirus B19: general considerations and impact on patients with sickle-cell disease and thalassemia and on blood transfusions. FEMS Immunol Med Microbiol. 2011;62: 247-262.  
  6. Kaiser PO, Riess T, O'Rourke F, Linke D, Kempf VA. Bartonella spp.: throwing light on uncommon human infections. Int J Med Microbiol. 2011;1: 7-15.  PMid:20833105  
  7. MagalhŃes RF, Cintra ML, Barjas-Castro ML, Del Negro GM, Okay TS, Velho PE. Blood donor infected with Bartonella henselae. Transfus Med. 2010;20: 280-282.  PMid:20345384  
  8. Maggi RG, Mascarelli PE, Pultorak EL, Hegarty BC, Bradley JM, Mozayeni BR, Breitschwerdt EB. Bartonella spp. bacteremia in high-risk immunocompetent patients. Diagn Microbiol Infect Dis. 2011;71: 430-437.  PMid:21996096 

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